Immune-Mediated Hematological Disorders as Uncommon Presentation Forms of Systemic Lupus Erythematosus

Abstract

Background: It is well known that immune-mediated hematological disorders can precede, be concurrent with or follow a systemic lupus erythematosus (SLE) diagnosis. Some are relatively frequent and even consist in diagnosis criteria for this rheumatic disease. However, others are uncommonly seen as a presentation form of an SLE.Objectives: To describe immune-mediated hematological disorders which are uncommonly presentation forms of systemic lupus erythematosus (SLE). Material & Methods:A retrospective study was carried out of patients showing immune-mediated hematological disorders uncommonly described as a presentation form of SLE.Results: Six hematology patients were studied: four diagnosed with Evans syndrome (ES), one with thrombotic thrombocytopenic purpura (TTP), and one with adult-onset macrophage activation syndrome (MAS) as presentation forms of SLE. Two ES cases, the TTP and MAS cases were diagnosed concurrently with SLE. The other two ES cases preceded SLE by months or years. Some clinical, laboratory, and pathological features of these hematological disorders are discussed along with aspects of their association to SLE.Discussion: Besides existing relatively frequent immune-mediated hematological disorders which even consist in diagnosis criteria for SLE, other hematological diagnoses can also uncommonly be seen as a presentation form of this rheumatic disease. Conclusion:This paper reports three immune-mediated hematological disorders uncommonly seen at SLE presentation: ES, TTP, and adultonset MAS.